Published online May 26, 2020. doi: 10.12998/wjcc.v8.i10.1806
Peer-review started: December 29, 2019
First decision: March 24, 2020
Revised: April 10, 2020
Accepted: April 27, 2020
Article in press: April 27, 2020
Published online: May 26, 2020
Processing time: 147 Days and 13.7 Hours
Antiphospholipid syndrome (APS) or Hughes syndrome is an acquired thromboinflammatory disorder. Clinical criteria of APS diagnosis are large- and small-vessel thrombosis as well as obstetric problems; laboratory criteria are the presence of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin antibodies and anti-β2-glycoprotein-1). The presence of at least 1 clinical and 1 laboratory criterion allows definitive diagnosis of APS. Primary APS is diagnosed in patients without features of connective tissue disease; secondary APS is diagnosed in patients with clinical signs of autoimmune disease. A high frequency of catastrophic APS as well as a high tendency to evolve from primary APS to secondary syndrome during the course of lupus and lupus-like disease is a feature of pediatric APS. The most characteristic clinical presentation of APS in the pediatric population is venous thrombosis, mainly in the lower limbs, and arterial thrombosis causing ischemic brain stroke. Currently, no diagnostic criteria for pediatric APS exist, which probably results in an underestimation of the problem. Similarly, no therapeutic procedures for APS specific for children have yet been established. In the present literature review, we discussed data concerning APS in children and its role in cerebrovascular diseases, including pediatric arterial ischemic stroke, migraine and cerebral venous thrombosis.
Core tip: Antiphospholipid syndrome in children is a rare disorder. The most characteristic clinical presentation of antiphospholipid syndrome (APS) in the pediatric population is venous thrombosis and arterial thrombosis causing ischemic stroke. However, no diagnostic criteria for pediatric APS exist at present, which probably results in an underestimation of the problem. We discussed data concerning APS in children and its role in cerebrovascular diseases, including pediatric arterial ischemic stroke, migraine and cerebral venous thrombosis.